Article
Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency.
Human molecular genetics - 1 Oct 2013
Cornelius Nanna, Byron Colleen, Hargreaves Iain, Guerra Paula Fernandez, Furdek Andrea K, Land John, Radford Weston W, Frerman Frank, Corydon Thomas J, Gregersen Niels, Olsen Rikke K J
Abstract excerpt
Coenzyme Q10 (CoQ10) is essential for the energy production of the cells and as an electron transporter in the mitochondrial respiratory chain. CoQ10 links the mitochondrial fatty acid β-oxidation to the respiratory chain by accepting electrons from electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO). Recently, it was shown that a group of patients with the riboflavin responsive form of multiple...
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